Lawrence Opas

Rhabdomyolysis with severe hypernatremia

Three children with severe hypernatremia presented with profound generalized weakness and biochemical evidence of rhabdomyolysis and myoglobinuria. These findings in combination have not been previously reported, to our knowledge, in children with severe hypernatremia. Unusual complications included respiratory failure in one child and cardiac arrhythmias in two children. All three children had acute renal insufficiency; one required peritoneal dialysis.

Human Immunodeficiency Virus-Associated Kaposi’s Sarcoma in a Pediatric Renal Transplant Recipient

An 11-year-old boy developed Kaposis sarcoma and progressive T lymphocyte deficiency 5 years after cadaveric kidney transplantation for end-stage renal disease. He had received 17 individual red blood cell transfusions prior to and during transplantation in 1980. Human immunodeficiency virus (HIV) was cultured from blood in cerebrospinal fluid and HIV antibodies were detected with enzyme immunoassay and immunoblot techniques. The recipient of the donors other kidney was well and HIV antibody-negative. The patient was treated with etoposide with excellent although transient regression of tumor. Allograft function has remained stable despite minimal immunosuppressive therapy and the need for high-dose anticonvulsant therapy. This case represents the first pediatric patient with acquired immune deficiency syndrome (AIDS) and Kaposis sarcoma following kidney transplantation.

Systemic lupus erythematosus with acanthosis nigricans, hyperpigmentation, and insulin receptor antibody

Acanthosis nigricans, insulin receptor antibody, and systemic lupus erythematosus are associated in the potentially lethal syndrome of type B insulin resistance. Hyperpigmentation has been reported rarely, while glucose intolerance is common in these patients. We report an adolescent girl with acanthosis nigricans, hyperpigmentation, insulin receptor antibody, and systemic lupus erythematosus without glucose intolerance. Insulin resistance may be mild or transient in some patients with type B insulin resistance. Resolution of skin lesions was noted during therapy of SLE, and was associated with disappearance of insulin receptor antibody.

Redefining Quality in Medical Education Research: A Consumer's View

BACKGROUND Despite an explosion of medical education research and publications, it is not known how medical educator consumers decide what to read or apply in their practice. OBJECTIVE To determine how consumers of medical education research define quality and value. METHODS Journal of Graduate Medical Education editors performed a literature search to identify articles on medical education research quality published between 2000 and 2013, surveyed medical educators for their criteria for judging quality, and led a consensus-building workshop at a 2013 Association of American Medical Colleges meeting to further explore how users defined quality in education research. The workshop used standard consensus-building techniques to reach concept saturation. Attendees then voted for the 3 concepts they valued most in medical education research. RESULTS The 110 survey responses generated a list of 37 overlapping features in 10 categories considered important aspects of quality. The literature search yielded 27 articles, including quality indexes, systematic and narrative reviews, and commentaries. Thirty-two participants, 12 facilitators, and 1 expert observer attended the workshop. Participants endorsed the following features of education research as being most valuable: (1) provocative, novel, or challenged established thinking; (2) adhered to sound research principles; (3) relevant to practice, role, or needs; (4) feasible, practical application in real-world settings; and (5) connection to a conceptual framework. CONCLUSIONS Medical educators placed high value on rigorous methods and conceptual frameworks, consistent with published quality indexes. They also valued innovative or provocative work, feasibility, and applicability to their setting. End-user opinions of quality may illuminate how educators translate knowledge into practice.

Discontinuation of Antimicrobial Prophylaxis (AP) in Children With Spina Bifida: A Case Series Analysis.

Background Spina bifida increases the risk for urinary tract infections (UTI). Antimicrobial prophylaxis (AP) reduces symptomatic UTI’s but selects resistant organisms. Measures to ensure regular and complete emptying of the bladder combined with treatment of constipation reduce the risk for UTI. Objectives Demonstrate that close adherence to a catheterization regimen in children with spina bifida (Selective Treatment - ST) reduces the need for antimicrobial prophylaxis. Methods Case series analysis of pediatric spina bifida clinic patients where routine antimicrobial prophylaxis was replaced by clean-catch catheterization and daily bowel regimen (ST). Retrospective chart review of 67 children (mean entry age: 24 months, median age: 4 months; 32 Males, 35 Females) enrolled between 1986 - 2004. Mean follow-up was 128.6 months (range 3 - 257 months). Asymptomatic and symptomatic UTI incidences were noted on AP and ST protocols. Creatinine clearance at study entry and follow-up was calculated by the age appropriate method. A multivariable regression model with delta Glomerular Filtration Rate (GFR) as the dependent variable, independent sample t-test and Wilcoxon rank sum were performed with SAS v. 9.2. Results The mean number of infections while on AP was 8.7 (95% CI 5.72, 11.68) and was 1.0 on ST (95% CI 0.48, 1.43). 5 infections on the AP protocol required intravenous (IV) antibiotics due to resistance to oral therapy, but none on ST. Comparing change in GFR between both protocols (AP vs. ST) found a significant difference in the change of GFR by treatment protocol. Conclusions AP did not prevent UTIs and resulted in more resistant organisms requiring IV antibiotics. Discontinuing AP allowed the return of susceptibility to oral antimicrobials and significantly improved GFR in those children who had previously been on AP. Adherence to a catheterization regimen with prompt treatment of symptomatic UTI conserved renal function and prevented selection of resistant organisms.

A 20-year-old woman with fatigue and palpitations.

Her blood pressure is 92/48 mm Hg, and her electrocardiogram shows abnormalities. What is going on?